Subepidermal autoimmune bullous diseases （SABD） are some autoimmune skin diseases that can present in a variety of forms and can be a challenging disease to treat. An overview of the different forms of SABD are disc...Subepidermal autoimmune bullous diseases （SABD） are some autoimmune skin diseases that can present in a variety of forms and can be a challenging disease to treat. An overview of the different forms of SABD are discussed including bullous pemphigoid （BP）, epidermolysis bullosa acquisita （EBA）, cicatricial pemphigoid （CP）, bullous systemic lupus erythematosus （BSLE）, and Anti-p200 pemphigoid. Emphasis on recent advancement is presented. In recent years, improved knowledge of the mechanisms of intercellular and cell-matrix adhesion has led to better understanding of the blistering process in some SABD. Defects of such structures cause the subepidermal bullous diseases and have also led to the discovery of new diseases （e.g. anti-p200-pemphigoid）. Recent studies have outlined the important role of autoantibodies, mast cell lymphocytes and their cytokines in pathogenesis of SABD.展开更多
Introduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature.Subepidermal autoimmune bullous diseases(SABD)are one of them which were not common.Case presentation:A 68-ye...Introduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature.Subepidermal autoimmune bullous diseases(SABD)are one of them which were not common.Case presentation:A 68-year-old male patient was admitted to our clinic with erosions on the oral mucosa,tense blisters,erosions,and ulcers on the trunk and extremities.Subepidermal vesicle formation was detected in the skin biopsy.Clinical examination revealed positivity for the Nikolsky phenomenon.The disease was unresponsive to conventional treatments and dysphagia and hoarseness occurred.The patient was screened for malignancy due to his unresponsiveness to the treatments and his severe oral mucosal involvement.Ig-G MGUS was detected in the patient.Discussion:The Nikolsky sign is an indicator of acantholysis and is known as a specific finding for pemphigus.However,when we look at gammopathy-associated autoimmune bullous dermatoses,skin fragility has been reported in cases.However,the meaning of fragility is not explained.The diagnosis of all these patients was Ig-M MGUS.Our patient was presented because of non-IgM MGUS,direct Nikolsky positivity,and severe mucosal involvement.Conclusion:Nikolsky positivity may be a clue for gammopathy-related subepidermal autoimmune bullous diseases.展开更多
Objective:Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium.The global distribution of Pemphigus varies according to genetic,...Objective:Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium.The global distribution of Pemphigus varies according to genetic,ethnic,socioeconomic,and cultural backgrounds.The purpose of our study is to evaluate the epidemiological features of pemphigus a single center in Morocco and compare our results with those reported elsewhere.Methods:A retrospective analysis was conducted of 302 pemphigus patients seen between 1990 and 2020 in the Dermatology Department of Ibn Sina Hospital(Rabat,Morocco).We further collected all the Moroccan scientific researches published by now to compare.Results:The average annual incidence was 0.32/100,000 inhabitants.The incidence doubled to 0.72 in 2020.The most common variant was pemphigus vulgaris(125 cases)followed by pemphigus erythematosus(99 cases),pemphigus foliaceous(40 cases),and vegetans(27 cases).The female to male ratio was 0.75,the average age at onset was 53 years old and the mean duration of the disease before diagnosis was 13.36months.Conclusion:This study joins the main characteristics of pemphigus in the Maghreb and around the world(pemphigus vulgaris most frequent subtype).In 2020,an epidemiological peak occurred during the coronavirus disease 2019 pandemic;probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2.展开更多
文摘Subepidermal autoimmune bullous diseases （SABD） are some autoimmune skin diseases that can present in a variety of forms and can be a challenging disease to treat. An overview of the different forms of SABD are discussed including bullous pemphigoid （BP）, epidermolysis bullosa acquisita （EBA）, cicatricial pemphigoid （CP）, bullous systemic lupus erythematosus （BSLE）, and Anti-p200 pemphigoid. Emphasis on recent advancement is presented. In recent years, improved knowledge of the mechanisms of intercellular and cell-matrix adhesion has led to better understanding of the blistering process in some SABD. Defects of such structures cause the subepidermal bullous diseases and have also led to the discovery of new diseases （e.g. anti-p200-pemphigoid）. Recent studies have outlined the important role of autoantibodies, mast cell lymphocytes and their cytokines in pathogenesis of SABD.
文摘Introduction:Numerous dermatoses associated with monoclonal gammopathy have been reported in the literature.Subepidermal autoimmune bullous diseases(SABD)are one of them which were not common.Case presentation:A 68-year-old male patient was admitted to our clinic with erosions on the oral mucosa,tense blisters,erosions,and ulcers on the trunk and extremities.Subepidermal vesicle formation was detected in the skin biopsy.Clinical examination revealed positivity for the Nikolsky phenomenon.The disease was unresponsive to conventional treatments and dysphagia and hoarseness occurred.The patient was screened for malignancy due to his unresponsiveness to the treatments and his severe oral mucosal involvement.Ig-G MGUS was detected in the patient.Discussion:The Nikolsky sign is an indicator of acantholysis and is known as a specific finding for pemphigus.However,when we look at gammopathy-associated autoimmune bullous dermatoses,skin fragility has been reported in cases.However,the meaning of fragility is not explained.The diagnosis of all these patients was Ig-M MGUS.Our patient was presented because of non-IgM MGUS,direct Nikolsky positivity,and severe mucosal involvement.Conclusion:Nikolsky positivity may be a clue for gammopathy-related subepidermal autoimmune bullous diseases.
文摘Objective:Pemphigus is a life threatening autoimmune bullous disease which involves the skin and mucous membranes of the stratified squamous epithelium.The global distribution of Pemphigus varies according to genetic,ethnic,socioeconomic,and cultural backgrounds.The purpose of our study is to evaluate the epidemiological features of pemphigus a single center in Morocco and compare our results with those reported elsewhere.Methods:A retrospective analysis was conducted of 302 pemphigus patients seen between 1990 and 2020 in the Dermatology Department of Ibn Sina Hospital(Rabat,Morocco).We further collected all the Moroccan scientific researches published by now to compare.Results:The average annual incidence was 0.32/100,000 inhabitants.The incidence doubled to 0.72 in 2020.The most common variant was pemphigus vulgaris(125 cases)followed by pemphigus erythematosus(99 cases),pemphigus foliaceous(40 cases),and vegetans(27 cases).The female to male ratio was 0.75,the average age at onset was 53 years old and the mean duration of the disease before diagnosis was 13.36months.Conclusion:This study joins the main characteristics of pemphigus in the Maghreb and around the world(pemphigus vulgaris most frequent subtype).In 2020,an epidemiological peak occurred during the coronavirus disease 2019 pandemic;probably related to stress and delayed time consultation for fear of contracting the severe acute respiratory syndrome coronavirus 2.
